Article

Pain Experience, Physical Function, Pain Coping, and Catastrophizing in Children With Sickle Cell Disease Who Had Normal and Abnormal Sensory Patterns

Authors:
To read the full-text of this research, you can request a copy directly from the authors.

Abstract

Introduction Sickle cell disease (SCD) is associated with recurrent pain that could lead to abnormal sensory patterns. The purpose of this study is to compare children with SCD who had normal sensory patterns (NSP) and abnormal sensory patterns (ASP) in pain experience, physical function, pain coping, and pain catastrophizing. Methods Children with Quantitative Sensory Testing data were selected from a larger study that examined pain and symptoms in children with SCD. Comparisons were made between children with NSP (n=35; 13.9 ± 1.9 years) and ASP (n=13; 12.8 ± 1.9 years). Children completed the Adolescent Pediatric Pain Tool (APPT), Functional Disability Index (FDI), Pain coping Questionnaire (PCQ), and Pain Catastrophizing Scale (PCS). Results No significant differences were found in pain intensity (2.9 ± 3.0 vs 2.6 ± 2.8 on 0 to 10 Visual Analog Scale) between the NSP and ASP, respectively. The most common marked pain sites for both groups were lower extremities (22.9%), head and neck (20.8%) and upper extremities (20.8%). Functional Disability Index scores were significantly worse in ASP (38.5%) compared to NSP (11.4%). The ASP group had significantly worse scores in emotion focused pain coping subscales. Conclusion Children with SCD with ASP had worse functional disability, were expressing more affective pain quality, and had emotion focused pain coping compared to NSP. Future studies are needed to examine the effectiveness of physical activities on the physical function, as well as psychosocial interventions such as peer support and creative arts expression to minimize development of ASP in children with SCD.

No full-text available

Request Full-text Paper PDF

To read the full-text of this research,
you can request a copy directly from the authors.

... Sözel olarak ifade edemedikleri için çoğu çocukta ağrının değerlendirilmesi güçtür. İnvaziv girişimler ya da ameliyat sonrası oluşan ağrılar çocuklarda daha yaygınken kronik hastalıklara bağlı batın, kas iskelet ve baş ağrıları da görülebilmektedir Hyacinth et al., 2020). ...
Article
Full-text available
The purpose of this study was to evaluate and identify the health-seeking behaviors, its associated factors, and the relationship between these factors to the health seeking behavior of young adults in Barangay Quiot Pardo, Cebu City. A sample size 332 respondents participated in the study. All the respondents were able to meet the inclusion criteria. The respondents were given likert-scale questionnaires online. Data were analyzed using SPSS. The findings of this study show that out of the 332 respondents, only 116 of those have sought treatment for their disease in the past 12 months. From the 332 young adults who participated in this study, the results showed that psychological, individual, and socio-cultural and familial factors have a significant relationship to the individual's decision to seek treatment during the period of disease while situational and marketing factors show no relationship to young adults' overall health-seeking behavior. The researchers conclude that psychological, individual, sociocultural and familial factors influence the health-seeking behaviors among young adults aging 20 to 24 years old residing in Barangay Quiot Pardo, Cebu City. The situational and marketing factors are less likely to affect the respondents' behavior.
... 6,7 Standard treatment for VOC includes hydration, as well as nonsteroidal anti-inflammatory drugs and opioid pain medications. 8,9 Although most studies show that opioids provide short-term relief, they are associated with side effects including constipation, opioid-induced hyperalgesia, and risk of addiction. 10 Numerous researchers have examined CIM in adults with SCD. ...
... 124,125 Endocrine and autonomic nervous system alterations in SCD pain require further exploration. Last, the role of motor/ physical function 126,127 and stress (or biological markers of stress) [128][129][130] in SCD continues to be studied, although the literature in this area is also not robust. ...
Article
Full-text available
Purpose of Review Sickle cell disease (SCD) is an inherited hemoglobinopathy with potential life-threatening complications that affect millions of people worldwide. Severe and disabling acute pain, referred to as a vaso-occlusive crisis (VOC), is a fundamental symptom of the disease and the primary driver for acute care visits and hospitalizations. Despite the publication of guidelines for VOC management over the past decade, management of VOCs remains unsatisfactory for patients and providers. Recent Findings Acute SCD pain includes pain secondary to VOCs and other forms of acute pain. Distinguishing VOC from non-VOC pain may be challenging for both patients and clinicians. Further, although opioids have been the gold-standard for VOC pain management for decades, the current highest standard of care for all acute pain is a multimodal approach that is less dependent on opioids, and, instead incorporates analgesics and adjuvants from different mechanistic pathways. In this narrative review, we focus on a multimodal pharmacologic approach for acute SCD pain management and explore the evidence for existing non-opioid pharmacological adjuncts. Moreover, we present an explanatory model of pain, which is not only novel in its application to SCD pain but also captures the multidimensional nature of the SCD pain experience and supports the need for such a multimodal approach. This model also highlights opportunities for new investigative and therapeutic targets – both pharmacological and non-pharmacological. Summary Multimodal pain regimens that are less dependent on opioids are urgently needed to improve acute pain outcomes for individuals with SCD. The proposed explanatory model for SCD pain offers novel opportunities to improve acute pain management for SCD patients.
... Additionally, children with SCD have been shown to experience pain that affects sleep patterns and how they cope with pain (e.g., behavioural distraction or catastrophising) (Graves and Jacob, 2014). However, it has also been shown that patients with SCD with and without abnormal sensory patterns (e.g., enhanced pain sensitivity) frequently use positive approaches (e.g., seeking social support) for coping with pain (Hyacinth et al., 2020). ...
Article
Full-text available
Sickle cell disease (SCD) refers to a group of inherited blood disorders with considerable morbidity that causes severe pain, reduces life expectancy, and requires significant self-management. Acute painful episodes are the hallmark of SCD, but persistent daily pain is also highly prevalent in this population. Characterising the impact and experience of SCD-related morbidity (i.e., sleep disruption, frequent emergency department visits, cognitive dysfunction) on health-related quality of life (HRQOL) requires multiple assessment methods to best capture the underlying mechanisms. To gain a greater understanding of the effect of common symptom categories on HRQOL and to determine potential pain coping targets, the present study investigated whether demographic, socioeconomic, sleepiness, pain burden, frequency of emergency department (ED) visits, and cognition predicted HRQOL in a paediatric sample of patients with SCD. Our study was a secondary analysis of baseline assessment data of children with SCD aged 8–15 years (n = 30) in the Prevention of Morbidity in Sickle Cell Anaemia Phase 2b (POMSb2) randomised controlled clinical trial of auto-adjusting continuous positive airways pressure. Patients completed cognitive testing (IQ, Processing Speed Index, Delis-Kaplan Executive Function Scale (DKEFS) Tower, Conner's Continuous Performance Test), sleepiness (Epworth Sleepiness Scale), and HRQOL (PedsQL Sickle Cell Module) at baseline. Patients reported pain burden (Sickle Cell Pain Burden Inventory-Youth) each month over 8 visits. Caregivers provided demographic information and reported their child's executive function (Behavioural Rating Inventory of Executive Function) at baseline. Data from our analysis demonstrated that demographic factors (i.e., age, gender, level of neighbourhood deprivation) and treatment variables (i.e., hydroxyurea use) did not independently predict HRQOL, and laboratory values (i.e., haemoglobin, haematocrit, mean oxygen saturation) were not significantly correlated with HRQOL (ps > 0.05). However, sleepiness, pain burden, ED visits, and executive dysfunction independently predicted HRQOL (R² = 0.66) with large effects (η² = 0.16 to 0.32). These findings identify specific, measurable symptom categories that may serve as targets to improve HRQOL that are responsive to change. This knowledge will be useful for multimodal interventions for paediatric patients with SCD that include sleep management, pain coping strategies, and executive function training.
Article
Sickle cell anemia (SCA) is a severe form of sickle cell disease that primarily affects black populations and individuals in tropical countries. This condition causes significant morbidity and mortality and leads to a range of psychosocial challenges. A preliminary search was conducted on Ovid Medline and public databases with a combination of Medical Subject Headings keywords, resulting in 368 articles. The articles were screened based on the selection criteria in a nonsystematic method by 3 researchers, and a narrative synthesis was done to analyze extracted data from selected peer-reviewed article. Mental disorders, sleep disturbances, interpersonal relationship challenges, stigmatization, and workplace discrimination were identified as significant contributors to the psychosocial distress experienced by individuals with SCA and their families. Depression and anxiety were prevalent among individuals with SCA, leading to poor treatment adherence, increased pain, and disruptions in various aspects of life. Sleep disturbances, including sleep-disordered breathing and sleepwalking, were also identified as significant contributors to poor sleep quality in SCA patients. Families of individuals with SCA also face challenges, including psychological stress, financial strain, and social disruption. Stigmatization is common, leading to misconceptions and discrimination. Workplace discrimination is prevalent, with a high unemployment rate among adult SCA patients. Comprehensive care is crucial to address these psychosocial issues. Early identification and intervention, comprehensive support programs, patient and family education, enhanced pain management strategies, and integration of mental health into clinical care are recommended. School-based support, research and advocacy, and community support groups are also important. By addressing these challenges through comprehensive care and support, healthcare professionals, policymakers, and society can reduce psychosocial distress and improve the lives of individuals with SCA.
Article
Objectives Over 50% of adults living with sickle cell disease have chronic pain, but the underlying mechanisms of chronic pain in this population remain unclear. Quantitative sensory testing is an important measurement tool for understanding pain and sensory processing. This scoping review summarizes quantitative sensory testing methodologies used in sickle cell studies and the evidence for central sensitization in this population. Methods We conducted a systematic search of PubMed, Embase, and CINAHL to identify studies using quantitative sensory testing in individuals living with sickle cell disease. Search strategies were based on variations of the terms “sickle cell disease,” and “quantitative sensory testing.” Eligible studies were observational or experimental studies in human participants living with SCD that reported findings and detailed methodology for at least one quantitative sensory testing modality. Results Our search yielded a total of 274 records; 27 of which are included in this scoping review. Of the 27 studies, 17 were original studies (with combined total of 516 adult and 298 pediatric participants) and 10 were secondary or subgroup analyses of these prior studies. Significant variation existed in quantitative sensory testing methodologies across studies, including testing locations, type and intensity of stimuli, and interpretation of findings. Of the identified studies, 22% (2/9 studies) reported sensory abnormalities in mechanical sensitivity and thresholds, 22% (2/9 studies) reported abnormal pressure pain thresholds, 46% (6/13 studies) reported sensory abnormalities in thermal pain thresholds and tolerance (cold and warm), and 50% (2/4 studies) reported abnormalities in temporal summation. Conclusion Future studies should use standardized quantitative sensory testing protocols with consistent and operationalized definitions of sensitization to provide clear insight about pain processing and central sensitization in sickle cell disease.
Article
Context: Structural racism negatively impacts individuals and populations. In the medical literature, including that of palliative care, structural racism's influence on interracial differences in outcomes remains poorly examined. Examining the contribution of structural racism to outcomes is paramount to promoting equity. Objectives: We examined portrayals of race and racial differences in outcomes in the palliative care literature and created a framework using critical race theory (CRT) to aid in this examination. Methods: We reviewed the CRT literature and iteratively developed a rubric to examine when and how differences between races are described. Research articles published in The Journal of Pain and Symptom Management presenting empiric data specifically including findings about racial differences were examined independently by three reviewers using the rubric. Results: Fifty-seven articles met inclusion criteria. Articles that specifically described racial differences were common in the topic areas of quality (75% of articles), hospice (53%), palliative care services (40%) and spirituality/religion (40%). The top three reasons posited for racial differences were patient preference (26%), physician bias (23%), and cultural barriers (21%). Using the CRT rubric we found that 65% of articles posited that a racial difference was something that needed to be rectified, while articles rarely provided narrative (5%) or other data on perspectives of people of color (11%) to explain assumptions about differences. Conclusions: Palliative care research frequently highlights racial differences in outcomes. Articles that examine racial differences often assume that differences need to be fixed but posit reasons for differences without the narratives of those most affected by them.
Article
Full-text available
Objective: To identify and synthesize evidence from randomized clinical trials that assessed the effects of music therapy on anxiety and depression symptoms in adults with mental disorders. Methods: This is a systematic review guided by the Preferred Reporting Items for Systematic Reviews and Meta- Analysis protocol. The search for articles in the MEDLINE, Embase, CENTRAL Cohcrane, CINAHL, PsycINFO and LILACS databases, as well as manual search, selection of studies, data extraction and methodological assessment using the Jadad Scale and the Cochrane risk-of-bias, was performed by two reviewers independently. Results: 1,649 studies were identified, eight of which were selected and analyzed. The findings showed a positive effect of music therapy on anxiety and depression symptoms in the adult population in different contexts. According to the Cochrane risk-of-bias, four studies were classified as low risk of bias, and the other four as high risk of bias. Adults who received music therapy showed physical and mental relaxation, reduced anxiety and depressive symptoms, promoting well-being in a conscious and healthy way. Conclusion: Music therapy presents itself as a potential strategy to reduce anxiety and depression as well as to help maintain and recover the health of adults with mental disorders. Keywords: Music therapy; Anxiety; Depression; Adult; Mental health
Article
Full-text available
Objective To evaluate evidence from randomised controlled trials and non-randomised controlled trials on the effectiveness of hospital clowns for a range of symptom clusters in children and adolescents admitted to hospital with acute and chronic conditions. Design Systematic review of randomised and non-randomised controlled trials. Data sources Medline, ISI of Knowledge, Cochrane Central Register of Controlled Trials, Science Direct, Scopus, American Psychological Association PsycINFO, Cumulative Index to Nursing and Allied Health Literature, and Latin American and Caribbean Health Sciences Literature. Study selection Randomised and non-randomised controlled trials were peer reviewed using the following eligibility criteria: children and adolescents who were admitted to hospital for acute conditions or chronic disorders, studies comparing use of hospital clowns with standard care, and studies evaluating the effect of hospital clowns on symptom management of inpatient children and adolescents as a primary outcome. Data extraction and synthesis Two investigators independently screened studies, extracted data, and appraised the risk of bias. Methodological appraisal was assessed by two investigators independently using the Jadad scale, the revised Cochrane risk-of-bias tool for randomised controlled trials (RoB 2), and the risk of bias in non-randomised studies (ROBINS-I) tool for non-randomised controlled trials. Results 24 studies (n=1612) met the inclusion criteria for data extraction and analysis. Most studies were randomised controlled trials (n=13). Anxiety was the most frequently analysed symptom (n=13), followed by pain (n=9), psychological and emotional responses and perceived wellbeing (n=4), stress (n=4), cancer related fatigue (n=3), and crying (n=2). Five studies used biomarkers, mainly cortisol, to assess stress or fatigue outcome following hospital clowns. Most of the randomised controlled trials (n=11; 85%) were rated as showing some concerns, and two trials were rated with a high risk of bias. Most non-randomised controlled trials (n=6; 55%) were rated with a moderate risk of bias according to ROBINS-I tool. Studies showed that children and adolescents who were in the presence of hospital clowns, either with or without a parent present, reported significantly less anxiety during a range of medical procedures, as well as improved psychological adjustment (P<0.05). Three studies that evaluated chronic conditions showed favourable results for the intervention of hospital clowns with significant reduction in stress, fatigue, pain, and distress (P<0.05). Conclusions These findings suggest that the presence of hospital clowns during medical procedures, induction of anaesthesia in the preoperative room, and as part of routine care for chronic conditions might be a beneficial strategy to manage some symptom clusters. Furthermore, hospital clowns might help improve psychological wellbeing in admitted children and adolescents with acute and chronic disorders, compared with those who received only standard care. Systematic review registration PROSPERO CRD42018107099.
Article
Full-text available
Purpose The study aimed to evaluate the effect of progressive muscle relaxation exercises on pain in patients with sickle cell disease. Materials-Method This randomized controlled interventional study was conducted in a hospital in Mersin, Turkey between October 2017 and July 2018. The study sample comprised 58 patients who were aged >18 years, conscious, had sickle cell disease, reported pain, and were treated with non-opioid or weak opioid analgesic based on the physician's recommendation (treatment group = 29, control group = 29). Data were collected by the individual presentation form and visual analog scale. During the study, both groups were treated with analgesics prescribed by the physician for three days. In addition to the analgesics, the treatment group was performed progressive muscle relaxation exercises for 30 minutes whereas the control group was rested. Pain level of both groups was evaluated at three time points every day for three days. In the study, frequency distributions and descriptive statistics were presented for categorical and numerical variables, respectively. Chi-square analysis, independent samples t-test, Mann–Whitney U test, three-way ANOVA, and Sidak test were used to analyze the difference between the variables. Results The mean age of the patients was 29.59 ± 6.94 years, and 53.4% of the patients were female and 69% were single. The mean pain score of the treatment group at the third time point on days 1, 2, and 3 was significantly lower than the control group (p < .05). There was no statistically significant difference between the two groups in terms of coping methods for pain, pain location, and complaints accompanying pain (p > .05). Conclusion Progressive muscle relaxation exercises were found to be effective in the pain management of patients with sickle cell anemia.
Article
Full-text available
Importance Individuals with sickle cell disease (SCD) have reduced life expectancy; however, there are limited data available on lifetime income in patients with SCD. Objective To estimate life expectancy, quality-adjusted life expectancy, and income differences between a US cohort of patients with SCD and an age-, sex-, and race/ethnicity-matched cohort without SCD. Design, Setting, and Participants Cohort simulation modeling was used to (1) build a prevalent SCD cohort and a matched non-SCD cohort, (2) identify utility weights for quality-adjusted life expectancy, (3) calculate average expected annual personal income, and (4) model life expectancy, quality-adjusted life expectancy, and lifetime incomes for SCD and matched non-SCD cohorts. Data sources included the Centers for Disease Control and Prevention, National Newborn Screening Information System, and published literature. The target population was individuals with SCD, the time horizon was lifetime, and the perspective was societal. Model data were collected from November 29, 2017, to March 21, 2018, and the analysis was performed from April 28 to December 3, 2018. Main Outcomes and Measures Life expectancy, quality-adjusted life expectancy, and projected lifetime income. Results The estimated prevalent population for the SCD cohort was 87 328 (95% uncertainty interval, 79 344-101 398); 998 were male and 952 were female. Projected life expectancy for the SCD cohort was 54 years vs 76 years for the matched non-SCD cohort; quality-adjusted life expectancy was 33 years vs 67 years, respectively. Projected lifetime income was 1227000foranindividualwithSCDand1 227 000 for an individual with SCD and 1 922 000 for a matched individual without SCD, reflecting a lost income of $695 000 owing to the 22-year difference in life expectancy. One study limitation is that the higher estimates of life expectancy yielded conservative estimates of lost life-years and income. The analysis only considered the value of lost personal income owing to premature mortality and did not consider direct medical costs or other societal costs associated with excess morbidity (eg, lost workdays for disability, time spent in the hospital). The model was most sensitive to changes in income levels and mortality rates. Conclusions and Relevance In this simulated cohort modeling study, SCD had societal consequences beyond medical costs in terms of reduced life expectancy, quality-adjusted life expectancy, and lifetime earnings. These results underscore the need for disease-modifying therapies to improve the underlying morbidity and mortality associated with SCD.
Article
Full-text available
Background: Medical procedures often evoke pain and anxiety in pediatric patients. Virtual reality (VR) is a relatively new intervention that can be used to provide distraction during, or to prepare patients for, medical procedures. This meta-analysis is the first to collate evidence on the effectiveness of VR on reducing pain and anxiety in pediatric patients undergoing medical procedures. Methods: On April 25, 2018, we searched EMBASE, MEDLINE, CENTRAL, PubMed, Web of Science, and PsycINFO with the keywords "VR", "children," and "gadolescents". Studies that applied VR in a somatic setting with participants ≤21 years of age were included. VR was defined as a fully immersive 3-dimensional environment displayed in surround stereoscopic vision on a head-mounted display (HMD). We evaluated pain and anxiety outcomes during medical procedures in VR and standard care conditions. Results: We identified 2889 citations, of which 17 met our inclusion criteria. VR was applied as distraction (n = 16) during venous access, dental, burn, or oncological care or as exposure (n = 1) before elective surgery under general anesthesia. The effect of VR was mostly studied in patients receiving burn care (n = 6). The overall weighted standardized mean difference (SMD) for VR was 1.30 (95% CI, 0.68-1.91) on patient-reported pain (based on 14 studies) and 1.32 (95% CI, 0.21-2.44) on patient-reported anxiety (based on 7 studies). The effect of VR on pediatric pain was also significant when observed by caregivers (SMD = 2.08; 95% CI, 0.55-3.61) or professionals (SMD = 3.02; 95% CI, 0.79-2.25). For anxiety, limited observer data were available. Conclusions: VR research in pediatrics has mainly focused on distraction. Large effect sizes indicate that VR is an effective distraction intervention to reduce pain and anxiety in pediatric patients undergoing a wide variety of medical procedures. However, further research on the effect of VR exposure as a preparation tool for medical procedures is needed because of the paucity of research into this field.This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives License 4.0 (CCBY-NC-ND), where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal.
Article
Full-text available
Background Sickle cell disease (SCD) is an inherited disorder of the red blood cells and is associated with chronic multisystem involvement. While SCD has been associated with poorer health-related quality of life (HRQoL), there is a paucity of data on the relationship of psychological covariates other than anxiety and depression and quality of life (QoL) in children with SCD. Materials and methods We performed a cross-sectional study of psychological factors, HRQoL, and pain-related outcomes in participants with SCD and race-matched controls as part of a larger study of experimental pain phenotyping. Results Pain catastrophizing was inversely correlated with HRQoL measured by the PedsQL™ Generic Core Scale in children with SCD, while this was not noted in control participants. Psychological factors, such as anxiety and depressive symptoms, were also associated with poorer HRQoL in both children with SCD and controls. We did not find an association of psychological factors with prior health care utilization. Psychological factors such as anxiety and depressive symptoms were inversely correlated with pain interference, but not pain intensity in SCD. Conclusion Catastrophizing is associated with poorer HRQoL in SCD, but in this study, it was not associated with pain intensity or interference and health care utilization in children with SCD. Further studies are needed to fully define the association of psychological factors including catastrophizing with QoL, pain burden, and SCD outcomes.
Article
Full-text available
Sickle cell disease (SCD) is a group of inherited disorders caused by mutations in HBB, which encodes haemoglobin subunit β. The incidence is estimated to be between 300,000 and 400,000 neonates globally each year, the majority in sub-Saharan Africa. Haemoglobin molecules that include mutant sickle β-globin subunits can polymerize; erythrocytes that contain mostly haemoglobin polymers assume a sickled form and are prone to haemolysis. Other pathophysiological mechanisms that contribute to the SCD phenotype are vaso-occlusion and activation of the immune system. SCD is characterized by a remarkable phenotypic complexity. Common acute complications are acute pain events, acute chest syndrome and stroke; chronic complications (including chronic kidney disease) can damage all organs. Hydroxycarbamide, blood transfusions and haematopoietic stem cell transplantation can reduce the severity of the disease. Early diagnosis is crucial to improve survival, and universal newborn screening programmes have been implemented in some countries but are challenging in low-income, high-burden settings. Full-text access to a view-only version is available by using the following SharedIt link: http://rdcu.be/I6qn
Article
Full-text available
Purpose: Opioid analgesics are commonly used to treat vaso-occlusive pain episodes in sickle cell disease (SCD), but comprehensive evidence characterizing opioid use in this patient population is limited. Our objective was to characterize opioid use patterns among SCD patients using a large nationwide database. Methods: A large, US medical claims database was utilized to identify a cohort of 3882 SCD patients, and characteristics of opioid use were analyzed. Clinical variables including age, gender, medication use, health care utilization, and medical history were evaluated for correlations with opioid use. Results: Forty percent of patients took opioid medications during a 12-month span, and the prevalence of any opioid use was highest for 20 to 29-year-old patients (58%). The median daily opioid dose was 1.85 mg (interquartile range: 0.62-10.68 mg) oral morphine equivalents (OME). While most opioid users took between 0 and 5 mg OME daily, 3% of pediatric patients and 23% of adult patients used more than 30-mg OME daily. High-dose opioid use was associated with older age, hydroxyurea therapy, nonsteroidal anti-inflammatory drug (NSAID) use, and frequent inpatient hospitalizations. In multivariable-adjusted analyses, patients with vaso-occlusive complications such as pain crisis (OR = 3.8, 95% CI 2.7-5.3) and avascular necrosis (AVN) (OR = 3.7, 95% CI 2.7-5.1) were associated with high-dose opioid use. Conclusions: Our study showed that only 40% SCD patients were on opioid analgesics during a 12-month span. However, a non-trivial number of patients used a much higher dose of opioids despite a relatively low average daily opioid dose among SCD patients, particularly with vaso-occlusive complications.
Article
Full-text available
Sickle cell disease is caused by an alteration in a single DNA base, but its clinical manifestations are influenced by other genes and behavioral and environmental factors. Recent findings may indicate an acceleration in the discovery of interventions that alter the disease course.
Article
Full-text available
Recent studies describe the clinical implications of sickle cell disease (SCD) stigma. However, little is known about its link to depressive symptoms or its relative influence on the association between depressive symptoms and SCD pain. We examined whether internalized stigma about SCD moderated the relation between depressive symptoms and pain among 69 adults attending a SCD clinic who reported pain episodes and health care use over the past 3 months. Unadjusted bivariate analyses showed a marginal association between depressive symptoms and SCD pain frequency (r = .21, p b = ?.10, p
Article
Full-text available
To the editor: Survival of patients with sickle cell disease (SCD) in high-income countries has improved greatly in the last 60 years. In 1960, it was described as a “disease of childhood”[1][1] whereas 25 years later, the Cooperative Study of Sickle Cell Disease reported that 85% of hemoglobin
Article
Full-text available
Chronic opioid therapy (COT) for chronic non-cancer pain is frequently debated, and its effectiveness is unproven in sickle cell disease (SCD). The authors conducted a descriptive study among 83 adult SCD patients and compared the severity of disease and pain symptoms among those who were prescribed COT (_n_=29) with those who were not using COT. All patients completed baseline laboratory pain assessment and questionnaires between January 2010 and June 2014. Thereafter, participants recorded daily pain, crises, function, and healthcare utilization for 90 days using electronic diaries. Analyses were conducted shortly after the final diary data collection period. Patients on COT did not differ on age, sex, or measures of disease severity. However, patients on COT exhibited greater levels of clinical pain (particularly non-crisis); central sensitization; and depression and increased diary measures of pain severity, function, and healthcare utilization on crisis and non-crisis diary days, as well as a greater proportion of days in crisis. Including depressive symptoms in multivariate models did not change the associations between COT and pain, interference, central sensitization, or utilization. Additionally, participants not on COT displayed the expected positive relationship between central sensitization and clinical pain, whereas those on COT demonstrated no such relationship, despite having both higher central sensitization and higher clinical pain. Overall, the results point out a high symptom burden in SCD patients on COT, including those on high-dose COT, and suggest that nociceptive processing in SCD patients on COT differs from those who are not.
Article
Full-text available
AimTo describe the perceptions of young adults with sickle cell disease concerning their disease experience.Background Sickle cell disease is a lifelong, genetic condition with both acute and chronic painful exacerbations. Little is known of the experiences of young adults with sickle cell disease.DesignThis study used a qualitative, descriptive design with semi-structured, life review interviews.Methods Between August 2010–September 2012, purposive sampling was used to recruit participants with a known sickle cell disease diagnosis who were ages 18–35 years, were being seen in an outpatient sickle cell clinic and were English speaking. Participants provided demographic information and responded to two interviews. A content analysis was then used to interpret participants’ narratives of their experiences of living with sickle cell disease.Results/findingsA sample of 29 young adults with sickle cell disease consisted of 79·3% females, 35·6% employed full-time or part-time, 71·6% single/never married and 57·8% with sickle cell anaemia. Their mean age was 25·8 with 13·2 years of education. Four major interview themes were identified: (1) struggles to maintain or achieve good quality of life or life satisfactions; (2) strategies to maintain self-care; (3) interruptions to family, work and social roles; and (4) difficulties accessing needed health care.Conclusion Young adults face many challenges while living with sickle cell disease. With a better understanding of their disease experience and how it influences their quality of life, researchers can begin tailoring appropriate interventions to improve health outcomes in this vulnerable, minority population.
Article
Full-text available
The majority of available studies have shown that children with sickle cell disease (SCD) have a higher risk of depressive symptoms than those without. The present study aimed to: assess the prevalence of depression in a sample of children with SCD; evaluate the association between disease severity, social support and depression, and the combined and/or singular effect on health-related quality of life (HRQL) in children with SCD; and show the predictive value of social support and disease severity on depression. A total of 120 children were included in the study, 60 (group I) with SCD and 60 matched, healthy control children (group II). Depression was assessed in both groups using the Children's Depression Inventory (CDI) and the Children's Depression Inventory-Parent (CDI-P). Children with CDI and CDI-P scores of more than 12 were interviewed for further assessment of depression using the Diagnostic Interview Schedule for Children Version IV (DISC-IV). The Pediatric Quality of Life Inventory Version 4.0 Generic Core Scales (PedsQL 4.0) was used to assess HRQL in both groups, and social support was measured with the Child and Adolescent Social Support Scale (CASSS). Eight (13%) of the 60 children with SCD had CDI and CDI-P scores of more than 12 (CDI mean score 14.50 ± 1.19, CDI-P mean score 14.13 ± 1.12), and were diagnosed as having clinical depression using the diagnostic interview DISC-IV. For group I, HRQL was poor across all PedsQL 4.0 domains in both self- and parent-reports (P < 0.001) compared with group II. A higher level of parent support was a significantly associated with decreased depressive symptoms, demonstrated by lower CDI scores. Better quality of life was shown by the associated higher total PedsQL 4.0 self-scores of children with SCD (B = -1.79, P = 0.01 and B = 1.89, P = 0.02 respectively). The present study demonstrates that higher levels of parent support were significantly associated with decreased depressive symptoms and better quality of life in children with SCD. Interventions focused on increasing parent support may be an important part of treatment for depression in children with SCD.
Article
Full-text available
Adults with sickle cell disease (SCD) report experiencing discriminatory behavior from some healthcare providers. The impact of discrimination on health outcomes in SCD, including adherence to physician recommendations, is not known.
Article
Full-text available
Very little is known about pain processing in sickle cell disease (SCD). We examined the mechanical and thermal sensory patterns in children with SCD. Children aged 10 to 17 years (n=48; mean 13.7±2.0 y; 22 females) participated in quantitative sensory testing (QST) procedures and completed a quality of life (PedsQL) and anxiety and depression scale (RCADS). Thirteen children showed evidence of abnormal pain processing, indicated by decreased sensitivity to heat or cold sensations (hypoesthesia), and pain experienced with nonpainful stimuli (allodynia). Pain ratings associated with cold and warm sensations were significantly higher in the subgroup with abnormal QST compared with the 35 SCD children with normal QST (P=0.01 and P<0.0001, respectively). The presence of hypoesthesia and allodynia in children with SCD may represent abnormal changes in the peripheral and central nervous system. Clinicians need to be aware that sickle cell pain may not only be inflammatory or ischemic secondary to vasoocclusion and hypoxia, but may also be neuropathic secondary to nerve injury or nerve dysfunction. Neuropathic pain in SCD may be the result of tissue damage after vaso-occlusion in neural tissues, whether peripherally or centrally. Future studies are needed to determine the presence of neuropathic pain in children with SCD.
Article
Full-text available
Perceived discriminatory experiences in society have been associated with a higher burden of pain among some minority patient populations. To describe the extent to which patients with sickle cell disease (SCD) perceive discrimination from health care providers, and to examine the association of these experiences with the burden of chronic SCD pain. Cross-sectional analysis of data collected at baseline of a prospective cohort study of SCD patient experiences of care (n = 291). Perceived race-based and disease-based discrimination from health care providers were measured using subscales adapted from the Interpersonal Processes of Care Survey. Discrimination scores were examined for their association with patient characteristics and measures of pain burden using descriptive, bivariate, and multivariate analytic techniques. Respondents reported a greater burden of race-based discrimination from health care providers than has been previously reported by African Americans, and they reported a greater amount of disease-based versus race-based discrimination. While age and having difficulty persuading providers about pain were the only patient characteristics independently associated with race-based discrimination, older age, greater emergency room utilization, having difficulty persuading providers about pain, daily chronic pain, fewer "good days" during a week, and a higher severity of pain on their "good days" were independently associated with greater disease-based discrimination. Perceived disease-based, but not race-based, discrimination was found to be associated with a greater range of self-reported pain among patients with SCD. If causal, this finding could signal an important new approach to mitigating the burden of pain experienced by persons with SCD.
Article
Full-text available
Background: The global burden of sickle cell anaemia (SCA) is set to rise as a consequence of improved survival in high-prevalence low- and middle-income countries and population migration to higher-income countries. The host of quantitative evidence documenting these changes has not been assembled at the global level. The purpose of this study is to estimate trends in the future number of newborns with SCA and the number of lives that could be saved in under-five children with SCA by the implementation of different levels of health interventions. Methods and findings: First, we calculated projected numbers of newborns with SCA for each 5-y interval between 2010 and 2050 by combining estimates of national SCA frequencies with projected demographic data. We then accounted for under-five mortality (U5m) projections and tested different levels of excess mortality for children with SCA, reflecting the benefits of implementing specific health interventions for under-five patients in 2015, to assess the number of lives that could be saved with appropriate health care services. The estimated number of newborns with SCA globally will increase from 305,800 (confidence interval [CI]: 238,400-398,800) in 2010 to 404,200 (CI: 242,500-657,600) in 2050. It is likely that Nigeria (2010: 91,000 newborns with SCA [CI: 77,900-106,100]; 2050: 140,800 [CI: 95,500-200,600]) and the Democratic Republic of the Congo (2010: 39,700 [CI: 32,600-48,800]; 2050: 44,700 [CI: 27,100-70,500]) will remain the countries most in need of policies for the prevention and management of SCA. We predict a decrease in the annual number of newborns with SCA in India (2010: 44,400 [CI: 33,700-59,100]; 2050: 33,900 [CI: 15,900-64,700]). The implementation of basic health interventions (e.g., prenatal diagnosis, penicillin prophylaxis, and vaccination) for SCA in 2015, leading to significant reductions in excess mortality among under-five children with SCA, could, by 2050, prolong the lives of 5,302,900 [CI: 3,174,800-6,699,100] newborns with SCA. Similarly, large-scale universal screening could save the lives of up to 9,806,000 (CI: 6,745,800-14,232,700) newborns with SCA globally, 85% (CI: 81%-88%) of whom will be born in sub-Saharan Africa. The study findings are limited by the uncertainty in the estimates and the assumptions around mortality reductions associated with interventions. Conclusions: Our quantitative approach confirms that the global burden of SCA is increasing, and highlights the need to develop specific national policies for appropriate public health planning, particularly in low- and middle-income countries. Further empirical collaborative epidemiological studies are vital to assess current and future health care needs, especially in Nigeria, the Democratic Republic of the Congo, and India.
Article
Full-text available
Purpose: This pilot study was conducted to determine the effectiveness of Healing Touch on anxiety, stress, pain, pain medication usage, and selected physiological measures of hospitalized adults with sickle cell disease experiencing a vaso-occlusive pain episode. Design: Healing Touch sessions were administered for 30 minutes on four consecutive days, and the self-reported data on anxiety, stress, pain, and the selected physiological data were collected while controlling for music and presence. Method: A parallel-group randomized control trial comparing the effects of Healing Touch with Music (HTM) to Attention Control with Music (ACM). Findings: Due to the small sample size, there were no statistically significant changes in any between-group comparisons, except for present pain on Day 4 for the ACM group. For both groups, the within-group comparison showed a nonsignificant reduction in physiological parameters, a statistically significant reduction in anxiety and stress for the ACM group after Day 4, and a statistically significant reduction in stress in the HTM group after Days 2 and 4. The pre- to postintervention reductions in present pain were greater in the HTM group across all 4 days, but the only statistically significant within groups findings were in the HTM group (p < .01) on Day 1. Conclusions: Further research is needed.
Article
Pain associated with sickle cell disease (SCD) is frequently treated with opioids which have many side effects. There is a need for adjuvant non-opioid therapies that can improve pain control. Acupuncture, an integrative approach, has been shown to be useful in non-SCD pain conditions but has been used to a very limited extent in SCD. In this report we present a single academic pediatric center experience showing acceptability, feasibility and improved pain experience with adjuvant acupuncture therapy in children with SCD and suggest that acupuncture should be explored as a treatment option for managing pain in SCD.
Article
Background Opioid analgesics are frequently used in the home setting to manage episodic pain in youth with sickle cell disease (SCD). Given the risk of adverse side effects, including constipation and sedation, understanding factors associated with at-home opioid use is important for maximizing pain relief while minimizing negative side effects. Purpose The present study aimed to evaluate the relationship between individual psychological factors (pain catastrophizing and negative affect), caregiver psychological factors (catastrophizing about child’s pain and caregiver negative affect), and home opioid use in youth with SCD. Methods Youth with SCD (n = 32) and a caregiver (n = 28) recruited during a routine outpatient hematology visit completed electronic 14 day diaries assessing pain, opioid use, and psychological factors. Results Approximately 28% of youth (n = 9) reported pain ≥50% of diary days and a third of youth (n = 11, 34%) used opioid analgesics at least one of the diary days. The number of days opioid analgesics were used ranged from 0 to 7 (50% of diary days). Results from generalized linear mixed models indicated greater child negative affect accounted for increased odds of opioid use on a given day when accounting for pain intensity. Greater caregiver catastrophizing about children’s pain was also associated with increased odds of children’s opioid use. Conclusions Child and parent psychological factors relate to child opioid use at home for SCD-related pain. Future research is warranted in larger samples to identify targets for interventions to enhance pain management while reducing opioid-related risk and side effects.
Article
Background: This is the first update of a review published in 2015, Issue 1. Chronic pain is common during childhood and adolescence and is associated with negative outcomes, such as increased severity of pain, reduced function, and low mood. Psychological therapies, traditionally delivered face-to-face with a therapist, are efficacious at reducing pain intensity and disability. To address barriers to treatment access, such as distance and cost of treatment, technology is being used to deliver these psychological therapies remotely. Therapies delivered remotely, such as via the Internet, computer-based programmes, and smartphone applications, can be used to deliver treatment to children and adolescents with chronic pain. Objectives: To determine the efficacy of psychological therapies delivered remotely compared to waiting list, treatment as usual, or active control treatments, for the management of chronic pain in children and adolescents. Search methods: We searched four databases (CENTRAL, MEDLINE, Embase, and PsycINFO) from inception to May 2018 for randomised controlled trials (RCTs) of remotely-delivered psychological interventions for children and adolescents with chronic pain. We searched for chronic pain conditions including, but not exclusive to, headache, recurrent abdominal pain, musculoskeletal pain, and neuropathic pain. We also searched online trial registries, reference sections, and citations of included studies for potential trials. Selection criteria: We included RCTs that investigated the efficacy of a psychological therapy delivered remotely via technology in comparison to an active, treatment as usual, or waiting-list control. We considered blended treatments, which used a combination of technology and up to 30% face-to-face interaction. Interventions had to be delivered primarily via technology to be included, and we excluded interventions delivered via telephone. We included studies that delivered interventions to children and adolescents (up to 18 years of age) with a chronic pain condition or where chronic pain was a primary symptom of their condition (e.g. juvenile arthritis). We included studies that reported 10 or more participants in each comparator arm, at each extraction point. Data collection and analysis: We combined all psychological therapies in the analyses. We split pain conditions into headache and mixed (non-headache) pain and analysed them separately. We extracted pain severity/intensity, disability, depression, anxiety, and adverse events as primary outcomes, and satisfaction with treatment as a secondary outcome. We considered outcomes at two time points: first immediately following the end of treatment (known as 'post-treatment'), and second, any follow-up time point post-treatment between three and 12 months (known as 'follow-up'). We assessed risk of bias and all outcomes for quality using the GRADE assessment. Main results: We found 10 studies with 697 participants (an additional 4 studies with 326 participants since the previous review) that delivered treatment remotely; four studies investigated children with headache conditions, one study was with children with juvenile idiopathic arthritis, one included children with sickle cell disease, one included children with irritable bowel syndrome, and three studies included children with different chronic pain conditions (i.e. headache, recurrent abdominal pain, musculoskeletal pain). The average age of children receiving treatment was 13.17 years.We judged selection, detection, and reporting biases to be mostly low risk. However, we judged performance and attrition biases to be mostly unclear. Out of the 16 planned analyses, we were able to conduct 13 meta-analyses. We downgraded outcomes for imprecision, indirectness of evidence, inconsistency of results, or because the analysis only included one study.Headache conditionsFor headache pain conditions, we found headache severity was reduced post-treatment (risk ratio (RR) 2.02, 95% confidence interval (CI) 1.35 to 3.01); P < 0.001, number needed to treat to benefit (NNTB) = 5.36, 7 studies, 379 participants; very low-quality evidence). No effect was found at follow-up (very low-quality evidence). There were no effects of psychological therapies delivered remotely for disability post-treatment (standardised mean difference (SMD) -0.16, 95% CI -0.46 to 0.13; P = 0.28, 5 studies, 440 participants) or follow-up (both very low-quality evidence). Similarly, no effect was found for the outcomes of depression (SMD -0.04, 95% CI -0.15 to 0.23, P = 0.69, 4 studies, 422 participants) or anxiety (SMD -0.08, 95% CI -0.28 to 0.12; P = 0.45, 3 studies, 380 participants) at post-treatment, or follow-up (both very low-quality evidence).Mixed chronic pain conditionsWe did not find any beneficial effects of psychological therapies for reducing pain intensity post-treatment for mixed chronic pain conditions (SMD -0.90, 95% CI -1.95 to 0.16; P = 0.10, 5 studies, 501 participants) or at follow-up. There were no beneficial effects of psychological therapies delivered remotely for disability post-treatment (SMD -0.28, 95% CI -0.74 to 0.18; P = 0.24, 3 studies, 363 participants) and a lack of data at follow-up meant no analysis could be run. We found no beneficial effects for the outcomes of depression (SMD 0.04, 95% CI -0.18 to 0.26; P = 0.73, 2 studies, 317 participants) and anxiety (SMD 0.53, 95% CI -0.63 to 1.68; P = 0.37, 2 studies, 370 participants) post-treatment, however, we are cautious of our findings as we could only include two studies in the analyses. We could not conduct analyses at follow-up. We judged the evidence for all outcomes to be very low quality.All conditionsAcross all chronic pain conditions, six studies reported minor adverse events which were not attributed to the psychological therapies. Satisfaction with treatment is described qualitatively and was overall positive. However, we judged both these outcomes as very low quality. Authors' conclusions: There are currently a small number of trials investigating psychological therapies delivered remotely, primarily via the Internet. We are cautious in our interpretations of analyses. We found one beneficial effect of therapies to reduce headache severity post-treatment. For the remaining outcomes there was either no beneficial effect at post-treatment or follow-up, or lack of evidence to determine an effect. Overall, participant satisfaction with treatment was positive. We judged the quality of the evidence to be very low, meaning we are very uncertain about the estimate. Further studies are needed to increase our confidence in this potentially promising field.
Article
1 Objective Due to incomplete management of vaso‐occlusive pain episodes (VOE) in patients with sickle cell disease (SCD), we sought to determine if immersive VR would be feasible for inpatients. Secondarily, we hypothesized that a single VR session would improve the VOE pain experience. 2 Procedures Consecutive patients with SCD eight years and older admitted for VOE were offered one 15‐minute VR session, utilizing a relaxing underwater world specifically created for pediatric patients and to minimize potential simulator side effects. Safety and acceptability were evaluated with a brief survey before and after the session. Pain was evaluated utilizing the validated adolescent pediatric pain tool (APPT). Survey data and pain scores were analyzed using Wilcoxon signed‐rank test as the data were nonnormally distributed. 3 Results Thirty patients, 21 female, with a median age of 16 years were enrolled, the majority having hemoglobin SS disease. The VR session had no reported side effects; all patients requested VR again in the future. Median pain intensity (pre‐VR 7.3 [interquartile range, IQR, 6.1, 8.8], post‐VR 5.8 [4.7, 7.9]), number of affected body areas (pre‐VR 3.0 [2.0, 7.8], post‐VR 2.0 [0, 4.8]), and qualitative measures including sensory, affective, evaluative, and temporal pain domains were all statistically reduced (i.e., P ≤0.01). 4 Conclusions VR therapy was feasible in a cohort of patients with SCD admitted for VOE. In addition to standard therapies, VR may help reduce the pain experience with SCD VOE. Further study is required to determine the impact of VR therapy on opioid usage and length of stay in hospital.
Article
Sickle cell disease (SCD) pain associates with cold temperature and touch. Patients and murine models with SCD have baseline thermal and mechanical pain. In SCD mice, the baseline hypersensitivity is exacerbated by experimental vaso-occlusive crises. We hypothesized that SCD patients will similarly experience increased hypersensitivity to thermal and mechanical stimuli during acute painful events compared to baseline health. We conducted a prospective study of 24 SCD patients ages 7-19 yrs. Patients underwent quantitative sensory testing to thermal (cold/heat) and mechanical stimuli on the thenar eminence of the non-dominant hand (glabrous skin) and the lateral dorsum of the foot (hairy skin) during baseline health and within 48 hrs of hospitalization for acute pain. Primary outcomes were changes in: 1) Cold Pain Threshold (°C), 2) Heat Pain Threshold (°C) and 3) Mechanical Pain Threshold (g). Median age was 10.5 (IQR 9-14.8) yrs, 67% were female and 92% were on hydroxyurea. SCD patients had increased cold pain sensitivity in the hand during hospitalization compared to baseline [25.2°C (IQR 18.4-27.5°C) vs. 21.3°C (IQR 4.9-26.2°C); p=0.011] and increased mechanical pain sensitivity in the foot during hospitalization [0.32g (IQR 0.09-1.1g) vs. 1.7g (IQR 0.4-8.3g); p=0.003]. There were no differences in heat pain sensitivity. The increased cold (p=0.02) and mechanical (p=0.0016) pain sensitivity during hospitalization persisted after adjusting for age, gender, hydroxyurea use, opioid consumption and numeric pain score. Thus, cold and mechanical pain is significantly worse during an acute SCD painful event as compared to baseline health in patients with SCD.
Article
Background Adults with sickle cell disease (SCD) experience acute pain that is multidimensional. Despite recent improvements in treatment, pain management remains a significant challenge for these individuals. Music therapy interventions have the potential to address several dimensions of SCD pain, but they require systematic investigation. Objective This study investigated feasibility and preliminary efficacy of a single-session electronic music improvisation with a music therapist to diminish pain intensity and improve pain relief and mood in adults with SCD. Methods Using a three-group mixed methods intervention design, we randomized 60 adults with SCD to standard care plus one of three 20-minute study conditions: 1) electronic music improvisation with a music therapist (MT); 2) recorded music listening (ML); or 3) no intervention (control). Measures of pain intensity (VASPI), pain relief (VASPR), and mood (VASMOOD) were assessed before and after the study conditions, with a subset of MT and ML participants interviewed after measure completion. Results Compared to control, MT produced significant improvements in VASPI (odds ratio (OR) = 5.12, P = 0.035) and VASMOOD (OR = 11.60, P = 0.005). ML produced significant improvements in VASMOOD compared to control (OR = 5.76, P = 0.040). Qualitatively, there were two prominent themes directly related to music: 1) ML and MT offered many positive and few negative effects; and 2) music therapists provided comfort beyond the music. Conclusions Preliminary findings were promising and support the need for additional studies evaluating improvisational music therapy interventions for acute pain management in adults with SCD.
Article
This is the protocol for a review and there is no abstract. The objectives are as follows: To determine the effectiveness of psychological therapies delivered remotely compared to waiting-list, treatment-as-usual, or active control for the management of chronic pain in children and adolescents.
Article
Background and aims: Pain is the hallmark of sickle cell anemia (SCA), presenting as recurrent acute events or chronic pain. Central sensitization, or enhanced excitability of the central nervous system, alters pain processing and contributes to the maintenance of chronic pain. Individuals with SCA demonstrate enhanced sensitivity to painful stimuli however central mechanisms of pain have not been fully explored. We hypothesized that adults with SCA would show evidence of central sensitization as observed in other diseases of chronic pain. Methods: We conducted a prospective study of static and dynamic quantitative sensory tests in 30 adults with SCA and 30 matched controls. Results: Static thermal testing using cold stimuli showed lower pain thresholds (p=0.04) and tolerance (p=0.04) in sickle cell subjects, but not for heat. However, SCA subjects reported higher pain ratings with random heat pulses (p<0.0001) and change in scores with temporal summation at the heat pain threshold (p=0.002). Similarly, with the use of pressure pain stimuli, sickle cell subjects reported higher pain ratings (p=0.04), but not higher pressure pain tolerance/thresholds or allodynia to light tactile stimuli. Temporal summation pain score changes using 2 pinprick probes (256 and 512mN) were significantly greater (p=0.004 and p=0.008) with sickle cell, and delayed recovery was associated with lower fetal hemoglobin (p=0.002 and 0.003). Conclusions: Exaggerated temporal summation responses provide evidence of central sensitization in SCA. Implications: The association with fetal hemoglobin suggests this known SCA modifier may have a therapeutic role in modulating central sensitization.
Article
Purpose: Sickle cell disease (SCD) significantly affects physical functioning. We examined physical activity (PA) patterns in children with SCD versus a national sample and factors associated with PA and participation in physical education and organized sports. Method: One hundred children with SCD completed a 58-item survey with questions from the 2009-2010 National Health and Nutrition Examination Survey (NHANES) Physical Activity Questionnaire and others on physical education and sports, disease impact, and physical functioning. Results: Compared to NHANES participants, more children with SCD (67 vs 42%, p < 0.01) reported doing at least 10 minutes of moderate-to-vigorous intensity PA (MVPA)/week. Children with SCD also reported spending more days in MVPA (2.3 vs. 1.4 days/week, p < 0.01). However, fewer reported spending ≥ 60 minutes/day in either vigorous PA (VPA) (24 vs. 43%, p = 0.01) or MVPA (17 vs 23%, p < 0.01). Additionally, 90% and 48% of children with SCD participated in physical education and sports, respectively. Greater disease impact on PA and physical functioning were associated with lower participation. Conclusion: Children with SCD are active at moderate to vigorous intensity for shorter durations. Negative personal beliefs about disease impact and poor physical functioning represent barriers to PA in SCD.
Article
Sickle cell disease is a common and life-threatening haematological disorder that affects millions of people worldwide. Abnormal sickle-shaped erythrocytes disrupt blood flow in small vessels, and this vaso-occlusion leads to distal tissue ischaemia and inflammation, with symptoms defining the acute painful sickle-cell crisis. Repeated sickling and ongoing haemolytic anaemia, even when subclinical, lead to parenchymal injury and chronic organ damage, causing substantial morbidity and early mortality. Currently available treatments are limited to transfusions and hydroxycarbamide, although stem cell transplantation might be a potentially curative therapy. Several new therapeutic options are in development, including gene therapy and gene editing. Recent advances include systematic universal screening for stroke risk, improved management of iron overload using oral chelators and non-invasive MRI measurements, and point-of-care diagnostic devices. Controversies include the role of haemolysis in sickle cell disease pathophysiology, optimal management of pregnancy, and strategies to prevent cerebrovascular disease.
Article
The relationships among depression, anxiety, and quality of life were tested, as were the effects of age, gender, and pain frequency on these variables in children (n = 44) and adolescents (n = 31) with sickle cell disease. Participants completed the Revised Child Anxiety and Depression Scale (ROADS) and the Pediatric Quality of Life (PedQL Generic Model). The mean and standard deviation for summary RCADS scores for the majority of participants were below the clinical thresholds of T < 65, indicating low risk for depression (n = 65; 89.3%) and anxiety (n = 70; 93.3%). The subscale scores for the different dimensions of QOL health were a) psychosocial (73.3 ± 15.9), b) emotional (75.0 ± 20.7), c) social (80.8 ± 19.1), d) school functioning (64.0 ≥ 19.8), and e) physical (77.4 ± 17.4). Significant negative correlations were found between mean total quality of life scores and symptoms of a) general anxiety (r = -0.51, p < 0.0001), b) depression (r = -0.66, p < 0.0001), c) obsessive compulsive (r = -0.53, p < 0.0001), d) panic (r = -0.60, p < 0.0001), and e) social phobia (r = -0.57, p < 0.0001). Age and gender did not have significant effects on risk for depression and anxiety or poor QOL. Pain frequency also did not have significant effects on the risk for depression and anxiety. Findings suggest that health care providers need to screen for anxiety and depression, and make referrals for early interventions to improve quality of life and promote school function in youth with sickle cell disease.
Article
Background: This study aims to examine the association of body image and weight perceptions with risk of depression and suicidal attempts in Jamaican adolescents with sickle cell disease (SCD). Methods: Adolescents with SCD and a national sample of Jamaican adolescents completed a questionnaire examining body image, weight perceptions, and risk for depression. Results: Perceived and desired body images were similar for both groups. Adolescents with SCD had higher levels of "negative body satisfaction" (43.9% vs. 33.9%; P = 0.03), risk for depression (28.7% vs. 19.3%; P = 0.01), and attempted suicide (12.4% vs. 6.6%; P = 0.02) than national sample. Risk of depression was higher in those who perceived themselves to be over or underweight, and lower in those with more friends and attending school. Females and those with body image dissatisfaction were more likely to have attempted suicide. Within the SCD adolescents, girls were at greater odds of having mental health issues. Conclusions: Jamaican adolescents with SCD have significantly higher rates of negative body satisfaction and depressive symptoms, and nearly twice the rate of attempted suicide, compared with their healthy peers. This underscores the need for healthcare professionals to better explore and discuss healthy weight, body satisfaction, and coping with the demands and uncertainties of having a chronic illness with Jamaican adolescents with SCD, even while promoting body acceptance and good self-esteem. Screening for mood disorders is strongly recommended and gender-specific interventions should be developed. Healthcare professionals need to encourage positive social interactions that improve adolescents' mental health.
Article
Purpose of review: Sickle cell disease (SCD) afflicts millions worldwide. The simplicity of its single nucleotide mutation belies the biological and psychosocial complexity of the disease. Despite only a single approved drug specifically for the treatment of SCD, new findings reviewed from 2015 provide the direction forward. Recent findings: The last year has provided a wealth of support for mechanisms affecting the red cell, hemolysis and vasculopathy, the innate immune system activation, blood cell and endothelial adhesiveness, central sensitization to pain, and chronic brain injury. The evidence supporting expanded use of hydroxyurea continues to mount. Many promising therapies are reaching clinical trial, including curative therapies, with more on the horizon. Summary: Evidence is compelling that the use of hydroxyurea must be expanded by clinicians to gain the full pleiotropic benefits of this approved drug. Clinicians must become aware that severe acute and chronic pain has a biological and neurologic basis, and the understanding of this basis is growing. Researchers are testing investigational therapies at an unprecedented pace in SCD, and partnership between patients, researchers, and the private sector provides the most rapid and productive way forward. Copyright
Article
Patients with sickle cell disease (SCD) display significantly lower mean/median thermal and mechanical pain thresholds compared with controls. This suggests impaired pain sensitivity where stimuli produce exaggerated pain. Despite these mean/median differences, clinicians need to understand if patients meet criteria for impaired pain sensitivity. We defined thresholds for impaired cold, heat, and mechanical pain sensitivity in SCD patients. Using quantitative sensory testing (QST) we assessed cold, heat, and mechanical pain thresholds in SCD patients and African American controls aged 7 years and above. Impaired pain sensitivity was defined as: (1) cold pain threshold 1 SD above control median threshold; (2) heat pain threshold 1 SD below control median threshold; and (3) mechanical pain threshold 1 SD below control median threshold. Fifty-five SCD patients and 57 controls participated in this study. Impaired pain sensitivity thresholds were: (1) cold: 17.01°C, (2) heat: 43.91°C, and (3) mechanical: 4.42 g. Impaired cold pain sensitivity was the most common finding (63.6%), then heat (60%), and mechanical (38.2%). Impaired pain sensitivity to ≥1 testing modalities occurred in 81.8% of SCD patients. Determining impaired pain sensitivity thresholds increases clinical utility of QST. QST could be a screening tool to phenotype SCD pain, an outcome for pain interventional trials, or guide pain neurobiology investigations.
Article
Context: Sickle cell disease (SCD) is a life-threatening condition that affects more than seven million people worldwide. The most common complication experienced by persons living with SCD is pain. Evidence supports the use of non-pharmacologic therapies in managing psychological and social complications of pain in persons with SCD, but there is little consensus if these approaches can also be applied for the treatment of pain in SCD. Objectives: To describe and synthesize the use of non-pharmacological therapies for reducing pain of any type and origin in persons SCD. Methods: A literature search was conducted using PsycINFO, PsycARTICLES, PubMed, CINAHL, and Embase. Databases were searched using the following terms: sickle cell, pain, and non-pharmacological therapies. Interventions were graded for methodological quality, and categorized as 1) peer-support group therapies, 2) educational/psychological therapies, and 3) skill-based therapies. Results: Twenty-eight non-pharmacological interventions for persons with SCD were examined. Of these studies, a wide variety of non-pharmacological interventions were tested. Twelve studies yielded significant improvements in pain, three studies reported no positive effect or differences between experimental and control conditions on pain or a pain-related outcome, and one study reported a negative or detrimental intervention effect. Conclusion: Approximately half of the studies reviewed demonstrated success in alleviating pain, suggesting that patients are able to use non-pharmacological interventions to reduce pain with some degree of success. Questions still remain regarding the efficacy and generalizability of these interventions for persons with SCD.
Article
Purpose: The aim of this study was to describe self-efficacy as a theoretical component of behavior change in various therapeutic treatments such as the management of SCD pain. Method: The participants were prepared to self-initiate the GI for 5 to 10 minutes three times each day regardless of pain and also during each pain episode. As part of the GI training a tape or CD with guided imagery messages was provided. Participants were monitored for 4 weeks pre and 4 weeks post intervention (GI training). Children kept a daily record of pain episodes. During this time, children continued to record as before in their personal study diary: pain episodes (intensity and treatment), school attendance, and also the frequency of GI use. At the conclusion of this 4-week period, usual pain patterns (PAT), visual imagery ability (KIAQ), and disease specific self-efficacy scale were measured again. The Sickle Cell Self-Efficacy Scale (SCSES) is a new nine-item scale measuring disease-specific perceptions of self-efficacy. The instrument's developers established internal consistency by Cronbach's alpha of 0.89. Results: H1: Children with SCD who are trained in guided imagery will have greater disease-specific self-efficacy following the training than they had prior to learning guided imagery; the hypothesis was tested and supported using t-tests of mean interval-level scores on the SCSES. Conclusion: Eighteen children had positive gained scores and sixteen children raised their scores more than one standard deviation above the mean score for this sample distribution. Greater self-efficacy scores are associated with better physical and psychological functioning.
Article
Background: Chronic pain is common during childhood and adolescence and is associated with negative outcomes such as increased severity of pain, reduced function (e.g. missing school), and low mood (e.g. high levels of depression and anxiety). Psychological therapies, traditionally delivered face-to-face with a therapist, are efficacious at reducing pain intensity and disability. However, new and innovative technology is being used to deliver these psychological therapies remotely, meaning barriers to access to treatment such as distance and cost can be removed or reduced. Therapies delivered with technological devices, such as the Internet, computer-based programmes, smartphone applications, or via the telephone, can be used to deliver treatment to children and adolescents with chronic pain. Objectives: To determine the efficacy of psychological therapies delivered remotely compared to waiting-list, treatment-as-usual, or active control treatments, for the management of chronic pain in children and adolescents. Search methods: We searched four databases (CENTRAL, MEDLINE, EMBASE, and PsycINFO) from inception to June 2014 for randomised controlled trials of remotely delivered psychological interventions for children and adolescents (0 to 18 years of age) with chronic pain. We searched for chronic pain conditions including, but not exclusive to, headache, recurrent abdominal pain, musculoskeletal pain, and neuropathic pain. We also searched online trial registries for potential trials. A citation and reference search for all included studies was conducted. Selection criteria: All included studies were randomised controlled trials that investigated the efficacy of a psychological therapy delivered remotely via the Internet, smartphone device, computer-based programme, audiotapes, or over the phone in comparison to an active, treatment-as-usual, or waiting-list control. We considered blended treatments, which used a combination of technology and face-to-face interaction. We excluded interventions solely delivered face-to-face between therapist and patient from this review. Children and adolescents (0 to 18 years of age) with a primary chronic pain condition were the target of the interventions. Each comparator arm, at each extraction point had to include 10 or more participants. Data collection and analysis: For the analyses, we combined all psychological therapies. We split pain conditions into headache and mixed (non-headache) pain and analysed them separately. Pain, disability, depression, anxiety, and adverse events were extracted as primary outcomes. We also extracted satisfaction with treatment as a secondary outcome. We considered outcomes at two time points: first immediately following the end of treatment (known as 'post-treatment'), and second, any follow-up time point post-treatment between 3 and 12 months (known as 'follow-up'). We assessed all included studies for risk of bias. Main results: Eight studies (N = 371) that delivered treatment remotely were identified from our search; five studies investigated children with headache conditions, one study was with children with juvenile idiopathic arthritis, and two studies included mixed samples of children with headache and mixed (i.e. recurrent abdominal pain, musculoskeletal pain) chronic pain conditions. The average age of children receiving treatment was 12.57 years.For headache pain conditions, we found one beneficial effect of remotely delivered psychological therapy. Headache severity was reduced post-treatment (risk ratio (RR) = 2.65, 95% confidence interval (CI) 1.56 to 4.50, z = 3.62,p < 0.01, number needed to treat to benefit (NNTB) = 2.88). For mixed pain conditions, we found only one beneficial effect: psychological therapies reduced pain intensity post-treatment (standardised mean difference (SMD) = -0.61, 95% CI -0.96 to -0.25, z = 3.38, p < 0.01). No effects were found for reducing pain at follow-up in either analysis. For headache and mixed conditions, there were no beneficial effects of psychological therapies delivered remotely for disability post-treatment and a lack of data at follow-up meant no analyses could be run. Only one analysis could be conducted for depression outcomes. We found no beneficial effect of psychological therapies in reducing depression post-treatment for headache conditions. Only one study presented data in children with mixed pain conditions for depressive outcomes and no data were available for either condition at follow-up. Only one study presented anxiety data post-treatment and no studies reported follow-up data, therefore no analyses could be run. Further, there were no data available for adverse events, meaning that we are unsure whether psychological therapies are harmful to children who receive them. Satisfaction with treatment is described qualitatively.'Risk of bias' assessments were low or unclear. We judged selection, detection, and reporting biases to be mostly low risk for included studies. However, judgements made on performance and attrition biases were mostly unclear. Authors' conclusions: Psychological therapies delivered remotely, primarily via the Internet, confer benefit in reducing the intensity or severity of pain after treatment across conditions. There is considerable uncertainty around these estimates of effect and only eight studies with 371 children contribute to the conclusions. Future studies are likely to change the conclusions reported here. All included trials used either behavioural or cognitive behavioural therapies for children with chronic pain, therefore we cannot generalise our findings to other therapies. However, satisfaction with these treatments was generally positive. Larger trials are needed to increase our confidence in all conclusions regarding the efficacy of remotely delivered psychological therapies. Implications for practice and research are discussed.
Article
Chronic pain is a major characteristic feature of sickle cell disease (SCD). The refractory nature of pain and the development of chronic pain syndromes in many patients with SCD suggest that central neural mechanisms contribute to pain in this disease. We used HbSS-BERK sickle mice, which show chronic features of pain similar to those observed in SCD, and determined whether sensitization of nociceptive neurons in the spinal cord contributes to pain and hyperalgesia in SCD. Electrophysiological recordings of action potential activity were obtained from single, identified dorsal horn neurons of the spinal cord in anesthetized mice. Compared to control HbAA-BERK mice, nociceptive dorsal horn neurons in sickle mice exhibited enhanced excitability as evidenced by enlarged receptive fields, increased rate of spontaneous activity, lower mechanical thresholds, enhanced responses to mechanical stimuli, and prolonged after-discharges following mechanical stimulation. These changes were accompanied by increased phosphorylation of mitogen activated protein kinases (MAPKs) in the spinal cord that are known to contribute to neuronal hyperexcitability, including c-Jun N-terminal kinase (JNK), p44/p42 extracellular signaling-regulated kinase (ERK), and p38. These findings demonstrate that central sensitization contributes to pain in SCD.
Article
Self-care is an important aspect of managing a chronic disease. In sickle cell disease (SCD), home self-care contributes to individual pain management and thus pain crisis prevention. A better understanding of self-care can help health care providers equip patients with the resources and skills necessary to participate in their disease management. The aim of this study was to examine factors that influence self-care among young adults with SCD. A descriptive, cross-sectional study was conducted using secondary data analysis. Participants were recruited from two SCD clinics in the southeastern United States. The sample consisted of 103 young adults, ages 18 to 30 years, with SCD. Bivariate correlations and regression analyses were used to evaluate the relationships among SCD self-efficacy, social support, sociodemographics, self-care, and hospital visits for pain crises. Study participants were primarily women (61.2%), unemployed or disabled (68%), lived with family (73.8%), and had an annual average of three hospital visits for pain crises. Participants, on average, had 12 years of education, an annual household income of $35,724, and were 24 years old. Social support (p = .001), SCD self-efficacy (p = .002), and years of education (p = .043) were significantly related to self-care. Of the hypothesized variables, only income was significantly associated with hospital visits for pain crises (r = −0.219, p = .05). Individuals with SCD may benefit from self-care interventions that enhance social support, SCD self-efficacy, and access to education. To inform intervention development, further investigation is needed regarding daily self-care behaviors used by young adults with SCD.
Article
Although neuropathic pain is increasingly recognized in sickle cell disease (SCD), it is unknown how neuropathic pain drugs are used in children with SCD. Thus, we investigated use of these drugs and hypothesized older age and female sex are associated with increased neuropathic drug use and the use of these drugs is associated with longer length of stay. We analyzed the Pediatric Health Information System (2004 to 2009) including all inpatient visits aged 0 to 18 years with any SCD-related (all genotypes) discharge diagnosis. To limit confounding we excluded psychiatric and seizure visits. Antiepileptics, tricyclic antidepressants, and selective serotonin reuptake inhibitors were drugs of interest. Generalized Estimating Equations determined the impact of age and sex on neuropathic drug use and the impact of neuropathic drug use on length of stay. We analyzed 53,557 visits; 2.9% received≥1 neuropathic drugs. The odds of receiving a neuropathic drug increased significantly with age (reference group, 0 to 4 y: 5 to 10, odds ratio [OR], 5.7; 11 to 14: OR, 12.5; 15 to 18: OR, 22.8; all P<0.0001] and female sex (OR, 1.5; P=0.001). Neuropathic drug use was associated with longer length of stay (risk ratio, 8.3; P<0.0001). Neuropathic drug use in children with SCD was associated with older age, female sex, and longer length of stay.
Article
Importance Sickle cell disease (SCD) is a life-threatening genetic disorder affecting nearly 100 000 individuals in the United States and is associated with many acute and chronic complications requiring immediate medical attention. Two disease-modifying therapies, hydroxyurea and long-term blood transfusions, are available but underused.Objective To support and expand the number of health professionals able and willing to provide care for persons with SCD.Evidence Review Databases of MEDLINE (including in-process and other nonindexed citations), EMBASE, Cochrane Database of Systematic Reviews, Cochrane Central Register of Controlled Trials, CINAHL, TOXLINE, and Scopus were searched using prespecified search terms and keywords to identify randomized clinical trials, nonrandomized intervention studies, and observational studies. Literature searches of English-language publications from 1980 with updates through April 1, 2014, addressed key questions developed by the expert panel members and methodologists.Findings Strong recommendations for preventive services include daily oral prophylactic penicillin up to the age of 5 years, annual transcranial Doppler examinations from the ages of 2 to 16 years in those with sickle cell anemia, and long-term transfusion therapy to prevent stroke in those children with abnormal transcranial Doppler velocity (≥200 cm/s). Strong recommendations addressing acute complications include rapid initiation of opioids for treatment of severe pain associated with a vasoocclusive crisis, and use of incentive spirometry in patients hospitalized for a vasoocclusive crisis. Strong recommendations for chronic complications include use of analgesics and physical therapy for treatment of avascular necrosis, and use of angiotensin-converting enzyme inhibitor therapy for microalbuminuria in adults with SCD. Strong recommendations for children and adults with proliferative sickle cell retinopathy include referral to expert specialists for consideration of laser photocoagulation and for echocardiography to evaluate signs of pulmonary hypertension. Hydroxyurea therapy is strongly recommended for adults with 3 or more severe vasoocclusive crises during any 12-month period, with SCD pain or chronic anemia interfering with daily activities, or with severe or recurrent episodes of acute chest syndrome. A recommendation of moderate strength suggests offering treatment with hydroxyurea without regard to the presence of symptoms for infants, children, and adolescents. In persons with sickle cell anemia, preoperative transfusion therapy to increase hemoglobin levels to 10 g/dL is strongly recommended with a moderate strength recommendation to maintain sickle hemoglobin levels of less than 30% prior to the next transfusion during long-term transfusion therapy. A strong recommendation to assess iron overload is accompanied by a moderate strength recommendation to begin iron chelation therapy when indicated.Conclusions and Relevance Hydroxyurea and transfusion therapy are strongly recommended for many individuals with SCD. Many other recommendations are based on quality of evidence that is less than high due to the paucity of clinical trials regarding screening, management, and monitoring for individuals with SCD.
Article
The current study aimed to validate the child and parent pain catastrophizing scale in a large chronic pain sample and to identify child pain catastrophizing clinical reference points. Patients and parents (n= 697) evaluated at a pediatric pain program completed the Pain Catastrophizing Scale, child (PCS-C) and parent (PCS-P) report, along with additional measures of psychological functioning. The measure's psychometric properties were examined, as well as relations across demographic, pain, and psychological characteristics and pain catastrophizing. Clinical reference points were identified for the PCS-C from differences in pain catastrophizing across levels of disability, depressive symptoms, and anxiety. Overall, we did not find support for the hypothesized three-dimension structure and recommend potentially removing items 7 and 8 for both the PCS-P and PCS-C due to floor/ceiling effects. The 11-item PCS-C is most parsimonious as a unitary construct, while the 11 item PCS-P is comprised of two factors. Although parent catastrophizing was significantly associated with child outcomes after controlling for pain level, it was no longer significant when accounting for child catastrophizing. When comparing PCS-C scores based on child outcomes, significant differences emerged for low, moderate, and high catastrophizing levels. It appears that the influence of parent catastrophizing on outcomes can be explained through its impact on child catastrophizing levels. Lastly, PCS-C reference points derived from this large sample can aid clinicians in assessment and treatment planning, in turn increasing the utility of the PCS-C for both clinical and research purposes.
Article
Purpose: The study examined the relationships among pain, pain coping, and sleep, and assessed factors (age, gender, frequency, and intensity of pain) that affect pain, coping, and sleep in children with sickle cell disease (SCD). Methods: Participants (66) were 39 children (M = 11.5 years) and 27 adolescents (M = 15.5 years) with SCD who completed an electronic visual analog scale (eVAS), Pain Coping Questionnaire, and Pittsburg Sleep Quality Index. Results: About two-thirds of the children reported pain the previous month. No significant differences were found between pain and age, gender, pain intensity, or frequency. Most children coped with pain by seeking information, problem solving, seeking social support, and positive self-statements. There were significant negative correlations in males between worse pain severity and behavioral distraction and internalizing or catastrophizing. The majority (91.2%) had mild to severe sleep disturbances, with 18.2% requiring sleeping medication three or more times a week. There were no significant differences between sleep and age, gender, pain intensity, or frequency. Conclusion: Children with SCD experience pain that affects sleep patterns and the way they cope with pain. Nurses need to concurrently assess pain, coping, and sleep and promote sleep hygiene and positive coping strategies during pain episodes.
Article
: Findings show the technique can be readily taught to and used effectively by this population. Despite innovations in treatment, disease-related pain is still the primary cause of hospitalization for children with sickle cell disease. Pharmacologic pain management relieves pain temporarily, but adverse effects are increasingly a concern. Cognitive behavioral therapies, which include the use of guided imagery, have shown promise in changing pain perception and coping patterns in people with chronic illnesses. Few studies have been done in children with sickle cell disease. The purposes of this study were to test the effects of guided imagery training on school-age children who had been diagnosed with sickle cell disease, and to describe changes in pain perception, analgesic use, self-efficacy, and imaging ability from the month before to the month after training. A quasi-experimental interrupted time-series design was used with a purposive sample of 20 children ages six to 11 years enrolled from one sickle cell disease clinic, where they had been treated for at least one year. Children completed pain diaries daily for two months, and investigators measured baseline and end-of-treatment imaging ability and self-efficacy. After training in the use of guided imagery, participants reported significant increases in self-efficacy and reductions in pain intensity, and use of analgesics decreased as well. Guided imagery is an effective technique for managing and limiting sickle cell disease-related pain in a pediatric population.
Article
This article presents the results of a 5-year formative evaluation study on sickle cell self-help groups. Sickle cell disease is a chronic genetic disease that primarily affects African Americans in the United States. The outcomes of this research provide an opportunity to discuss the participation of African Americans in self-help groups. This study is the first known to focus exclusively on self-help groups for African Americans. In 1992, contact was made with 134 groups representing 31 states, the District of Columbia, and one province of Canada. This represents all sickle cell groups known to exist in the United States. Data were collected on 123 of the 134 groups. The results show that 20% of the groups existed for more than 10 years, including one group that started 23 years ago. Since 1985, a steady, yearly growth rate of 13.4% has been calculated. Clearly, African Americans have been in and continue to join self-help groups. Continued research with these groups is needed to augment the current formative evaluation study.
Article
Very few multidimensional tools are available for measurement of pain in children and adolescents. We critically reviewed the scientific literature to examine the psychometrics and utility of the Adolescent Pediatric Pain Tool (APPT), a multidimensional self-report tool that evaluates the intensity, location, and quality (including affective, evaluative, sensory, and temporal) dimensions of pain. The APPT is available in English and Spanish for children and adolescents, and was modeled after the McGill Pain Questionnaire in adults. We found good evidence for construct validity, reliability, and sensitivity of the APPT for the measurement of pediatric pain. The APPT was used to measure pain in children with different conditions, such as cancer, sickle cell disease, orthopedic, traumatic injuries, and allergy testing. Although the APPT was designed to assess the multiple dimensions of pain, the majority of the reports included results only for the intensity ratings. Unlike the numerical and pediatric faces rating scales, which are widely used in clinical practice and research, the APPT is not limited to the single dimension of pain intensity. It measures multiple dimensions, and may be able to discriminate between nociceptive and neuropathic pain. The APPT is one of a few multidimensional pain measures that can help to advance the science of pediatric pain and its management. When the APPT is used in practice or research, the multiple dimensions of pain may be characterized and compared in different painful conditions. It may guide the use of multimodal interventions in children and adolescents with a variety of pain conditions.
Article
Objectives. To evaluate the immediate effectiveness of a cognitive-behavioural group therapy intervention for the management of sickle cell disease SCD pain. Design. This study is taken from an ongoing longitudinal, multi-centred, randomized controlled trial. Method. Ninety-seven patients with SCD recruited from seven London hospitals were randomly allocated to one of three treatment conditions after giving written consent: a CBT pain management group, an attention placebo group and a nointervention control. All participants completed a number of measures at baseline and post-intervention to assess psychological well-being, pain and coping. Results. Results are presented for the 59 participants who met the study entrance criteria, attended treatment group sessions above the cut-off point selected for treatment completion, and completed sessions both baseline and post-intervention assessments. Significant post-intervention treatment group differences in favour of the CBT intervention were identified for nearly all of the psychological measures using the Kruskal-Wallis test. Within-group significant differences over time were also found for the CBT condition for the majority of the measures. Following the identification of centre differences in baseline measures, two-way ANCOVAs were conducted with centre and condition for the ranked data of each psychological measure using ranked baseline scores as covariates. Observation of the adjusted means indicated that most of the post-intervention effect was accounted for by centres 1 and 2, with centre 4 having a minimal effect and, for centre 3, some of the measures showing a reverse effect to that predicted. Conclusions. Overall, the cognitive-behavioural approach employed appears to be immediately effective for the management of SCD pain in terms of reducing psychological distress and pain, and improving coping. Further analysis of longitudinal and cost-effectiveness data currently being collated will indicate whether long-term and financial benefits are to be gained.
Article
A cross-sectional exploratory study was conducted comparing outcomes for 36 adults with sickle cell disease (SCD) receiving treatment at a hospital in London. Standard measures for self-reported pain and health service utilization, quality of life, psychological coping, anxiety and depression were administered to 21 patients engaged in cognitive behavioural therapy (CBT), and 15 patients treated with hydroxyurea (a disease-modifying drug). Patients who received CBT reported more pain episodes, shorter hospital admissions, more improved general health perceptions, and utilized significantly more psychological coping methods for pain than patients treated with hydroxyurea. There were no differences in mood. We conclude that both CBT and hydroxyurea appear to help improve quality of life in patients with SCD. CBT seems to have the additional advantage of increasing psychological coping ability, and may therefore be beneficial adjunctive to hydroxyurea.
Article
Pain control can be central to the effective management of sickle cell disease (SCD). The traditional medical model has relied on pharmacological intervention as the primary treatment approach to pain management. Alternatively, psychologists have the capability of assisting people with SCD through methods that empower them to control SCD pain. The concepts advanced in this article are based on the clinical experiences of the authors in working with people who have SCD. The purpose of the article is to describe alternative strategies to the traditional pharmacological methods for management of SCD pain. The authors examine in more depth cognitive strategies and autonomic nervous system engaging strategies that have been found to be effective in the self-induced management of SCD pain.